Total anomalous pulmonary venous connection
- Brian Soriano, MD
Brian Soriano, MD
- Associate Professor of Pediatrics & Adjunct Associate Professor of Radiology
- Heart Center, Seattle Children’s Hospital
- University of Washington School of Medicine
- David R Fulton, MD
David R Fulton, MD
- Section Editor — Pediatric Cardiology
- Associate Professor of Pediatrics
- Harvard Medical School
Total anomalous pulmonary venous connection (TAPVC), also referred to as total anomalous pulmonary venous return (TAPVR), is a cyanotic congenital defect in which all four pulmonary veins fail to make their normal connection to the left atrium. This results in drainage of all pulmonary venous return into the systemic venous circulation.
The anatomic variants, clinical manifestations, diagnosis, and management of TAPVC will be reviewed here.
The incidence of total anomalous pulmonary venous connection (TAPVC) ranges from 0.6 to 1.2 per 10,000 live births [1,2]. Among patients born with congenital heart disease (CHD), the incidence of TAPVC ranges between 0.7 and 1.5 percent. It is the fifth most common cause of cyanotic CHD .
In normal development, the lung buds are formed from the primitive foregut and share a common vascular plexus (splanchnic plexus), which initially drains into the common cardinal and umbilicovitelline venous systems. With the formation of the lungs (27 to 29 days of gestation), a portion of the splanchnic plexus differentiates into the primitive pulmonary vascular bed . During this same time period, the primitive left atrium forms a primordial evagination (common pulmonary vein) that grows into and joins the pulmonary portion of the splanchnic plexus. Once the connection is made, the primitive pulmonary venous system separates from the cardinal and umbilicovitelline veins. Portions of the common pulmonary vein are subsequently incorporated into the wall of the left atrium and become the two right and two left pulmonary veins, each of which enters the left atrium through a separate orifice.
Total anomalous pulmonary venous connection (TAPVC) arises from the failure of the left atrium to link to the pulmonary venous plexus, which results in the retention of connections to the primitive cardinal and umbilicovitelline drainage systems. The anatomic variants of TAPVC are dependent upon which connections are retained. The cardinal venous system provides connections to the innominate vein, right atrium, superior vena cava, or azygous vein, and the umbilicovitelline system to the portal or hepatic vein, or inferior vena cava.
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- ANATOMIC VARIANTS
- Obstructed forms
- Unobstructed forms
- CLINICAL MANIFESTATIONS
- Physical examination
- Other congenital anomalies
- Chest radiography
- NATURAL COURSE
- Initial medical management
- FOLLOW-UP CARE
- INFORMATION FOR PATIENTS
- SUMMARY AND RECOMMENDATIONS